Patients with sickle cell disease (SCD) are at risk of neurocognitive deficits, and accessing neuropsychology services could help patients and their physicians identify and treat them, according to results of a new study published in the British Journal of Haematology.

The authors recommend a multidisciplinary collaboration between hematology and neuropsychology to improve symptoms and outcomes and facilitate further research on the impact of neurocognitive deficits in patients with SCD.

“Individuals with SCD are at risk for neurocognitive deficits across the lifespan and processing speed has emerged as a recurring area of weakness for individuals with SCD,” the researchers said. “Severe genotype, low hemoglobin and hematocrit, and cerebrovascular insult are associated with worse cognitive outcomes.”

Furthermore, neurocognitive problems have been associated with poorer instrumental activities of daily living skills, particularly the transition from pediatric to adult care, adherence to medication, and employment.

Read more about SCD comorbidities

On the other hand, symptomatic treatments for SCD, such as transfusion therapy and hydroxycarbamide, might offer neuroprotective benefits to patients. The authors reviewed studies that showed improvements in reading comprehension and full-scale intellectual quotient after 1 year of hydroxycarbamide treatment, suggesting that therapy might slow neurocognitive decline and that patients undergoing chronic transfusion therapy have improved executive function in the short term.

The authors also found that patients with SCD who had access to neuropsychology services benefited from improved access to special education, vocational training support, and fewer emergency room visits and hospitalizations.

In addition, early interventions for parents have been associated with improvements in cognition and language among infants and toddlers, and computer-based training has led to improvements in working memory in children with SCD. Thus, the research team concluded that incorporating neuropsychology into SCD treatment will provide a wide range of clinical, social, and economic benefits to patients.


Longoria JN, Heitzer AM, Hankins JS, Trpchevska A, Porter JS. Neurocognitive risk in sickle cell disease: utilizing neuropsychology services to manage cognitive symptoms and functional limitations. Br J Haematol. Published online February 4, 2022. doi:10.1111/bjh.18041